A 6-year-old, spayed female rottweiler was referred for left forelimb lameness followed by tetraparesis. A mast cell tumor compressing the spinal cord at the level of the sixth cervical to first thoracic (C6-T1) vertebrae was diagnosed based on cervical myelography and necropsy findings. This was considered a primary extracutaneous mast cell tumor, as no evidence of disease was found elsewhere. This is the first report of a primary mast cell tumor in this location.